Idiopathic Pulmonary Fibrosis

Vol. 20 •Issue 4 • Page 17
Idiopathic Pulmonary Fibrosis

Big Challenges Offer Opportunity for Future Researchers

“That is a step on which I must fall down, or else o’erleap, for in my way it lies.” —William Shakespeare, Macbeth

For years, respiratory therapists have been the go-to experts for lung diseases, especially mounting threats like COPD. And as the body of scientific evidence about obstructive pulmonary diseases has grown, caregivers have benefited. So have their patients.

Therapists have learned how to better manage the conditions and improve a COPDer’s quality of life and even extend it. However, the next challenge before the respiratory community is restrictive pulmonary disease, including idiopathic pulmonary fibrosis (IPF). And at this point, there is still much to be learned and gained.

“There is almost no evidence, but you are going to be seeing these patients,” Brian Carlin, MD, asserted at the AARC International Congress in Las Vegas in December. “In fact, I would venture a guess that you are seeing these patients on a daily basis now.”

Overviewing the few clinical trials that have been completed to date, he tried to offer RTs a sense of what conclusions can be drawn at this point. There’s not much in the way of medicine and therapy, for example.

“Oxygen, steroids and rehabilitation: these are the big three,” said Carlin, senior staff physician at Allegheny General Hospital in Pittsburgh.

Available treatments for these patients won’t halt the disease or offer a cure, noted IPF researcher Harold Collard, MD, coordinator of the Interstitial Lung Disease Program at San Francisco General Hospital.

“Things have changed over the past ten years in terms of how we think about this disease and approach therapy,” he explained. “The truth remains that we don’t have a good therapy for this condition, and that is frustrating.”

Just the Facts

IPF is a chronic and generally fatal interstitial lung disorder, one of about 200 included in the interstitial lung diseases (ILDs) group. They all involve inflammation and scarring of the interstitium or tissue layer between the alveoli of the lung and the blood vessels.

IPF is characterized by scarring that thickens and stiffens the interstitium, causing an irreversible loss of the tissues’ ability to transport oxygen. Patients with IPF typically experience shortness of breath and a dry cough, which become progressively worse and debilitating.

IPF is the deadliest form of pulmonary fibrosis, with a median survival time from diagnosis of three to five years, and a five-year survival rate of approximately 20 percent.

Although relatively rare, affecting about 128,000 people in the United States, IPF is on the rise, increasing 150 percent in the past six years, according the American Journal of Respiratory and Critical Care Medicine. Recent studies show the disease is 5 to 10 times more prevalent than previously thought, occurring more often among men than women.

Many ILDs have known causes such as exposure to asbestos or certain medications. However, the cause of IPF is unknown, and until recently, there were no uniform diagnostic standards for IPF.

Other diseases with similar symptoms have often been diagnosed as IPF, despite widely varying prognoses. In addition, IPF is known by a number of other names, and this fact has caused confusion for patients and physicians alike.

Some patients with IPF benefit from lung transplantation surgery. Still, approximately 30 percent of these patients succumb to the disease before they can receive a transplant, according to the United Network for Organ Sharing.

There are risks even after a successful transplantation. Current survival rates are as high as 80 percent at one year following transplantation and 60 percent at four years. The most critical period for survival of both the patient and the donor organs is the first year after transplantation. That’s when surgical complications, acute rejection and infection are the greatest threats.

Pulmonary rehabilitation, on the other hand, can aid all IPF patients. “I try to send every one of these patients for pulmonary rehab. It’s fantastic,” said Collard. “Unfortunately, this hasn’t been proven to help in restrictive lung diseases like it has in obstructive lung disease.”

Components of a successful rehab program for IPF are similar ones for COPD patients: good assessment, endurance and strength training and psychosocial support, possibly through community groups. Goals for physical exercise remain unclear at this point, according to Carlin.

“I would say exercise them at the same rate as someone with COPD. Endurance training is the primary element,” he said, adding that care should be tailored to each patient’s needs. “Set your goals with the patient, set the patient’s goals and determine what is important to them overall.”

“You may not be able to halt the disease progression, but you can improve quality of life,” Collard added.

For more information, check the Pulmonary Fibrosis Foundation Web site at or Coalition for Pulmonary Fibrosis at

Shawn Proctor, associate editor and Web editor, can be reached at [email protected].